Multi-organ investigation in 16 CADASIL families from central Italy sharing the same R1006C mutation.

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) may involve many target organs with relevant variability among affected individuals. We performed a multi-organ assessment tapping nervous system, skeletal muscle and cardiovascular system in thirty-nine individuals belonging to 16 families from Central Italy sharing the same R1006C CADASIL mutation. Stroke prevalence was larger in female patients (66.

An immunological analysis of dystroglycan subunits: lessons learned from a small cohort of non-congenital dystrophic patients.

The dystroglycan (DG) expression pattern can be altered in severe muscular dystrophies. In fact, some congenital muscular dystrophies (CMDs) and limb-girdle muscular dystrophies (LGMDs) are caused by point mutations identified in six glycosyltransferase genes which are likely to target different steps along the posttranslational "O-glycosylation route" leading to a fully decorated and functional α-DG subunit. Indeed, hypoglycosylation of α-DG is thought to represent a major pathological event, in that it could reduce the DG's ability to bind the basement membrane components, thus leading to sarcolemmal instability and necrosis.

Attachment dimensions and group climate growth in a sample of women seeking treatment for eating disorders.

Adult attachment and group process research are emerging areas of research for treating eating disorders. In this study, we examined several aspects of group processes: the weekly growth of group therapy climate, the relationship between group climate growth and outcomes, and the impact of the group on individual experiences of group climate. Further, we assessed the relationship between adult attachment dimensions and these group processes.

Internalization of the thin and muscular body ideal and disordered eating in adolescence: the mediation effects of body esteem.

This study investigates body esteem factors (weight-esteem and appearance-esteem) as mediators of the relationship between 'internalization of the ideal body figure' and disordered eating behaviors (restrained, emotional and external eating) in a community sample of adolescent males (n=810) and females (n=1137) from the Ontario Research on Eating and Adolescent Lifestyles (REAL) study. Mediation models were examined using a bootstrapping approach to test indirect effects and indirect contrasts. In males, weight-esteem partially mediated the relationship between muscular ideal and restrained eating; appearance-esteem partially mediated effects in the emotional and external eating regressions.

Interpersonal processes in psychodynamic-interpersonal and cognitive behavioral group therapy: a systematic case study of two groups.

This mixed method systematic case study applied an interpersonal stage model of the therapeutic process to examine interpersonal processes among a highly adherent Group Psychodynamic-Interpersonal Psychotherapy (GPIP) therapist and a highly adherent Group Cognitive Behavioral Therapy (GCBT) therapist and their groups of binge eating disordered (BED) patients. This is the first case study to apply the interpersonal stage model of psychotherapy to compare GCBT and GPIP methods and the first to apply the model to group therapy. Early-, middle-, and late-stage transcribed video recordings of sequential interactions among therapists and patients in each of these two time-limited group therapies were analyzed with the Structural Analysis of Social Behavior (SASB).

Predictors of treatment acceptance and of participation in a randomized controlled trial among women with anorexia nervosa.

Objective: This study aimed to identify predictors of acceptance of intensive treatment and of participation in a randomized controlled trial (RCT) among women with anorexia nervosa (AN).

Method: Participant data were drawn from a tertiary care intensive treatment programme including a previously published RCT. Women with AN (N = 106) were offered intensive treatment, and 69 were approached to participate in an RCT of olanzapine's efficacy as an adjunctive treatment for AN.

Implications of attachment theory and research for the assessment and treatment of eating disorders.

In this paper, we review the research literature on attachment and eating disorders and suggest a framework for assessing and treating attachment functioning in patients with an eating disorder. Treatment outcomes for individuals with eating disorders tend to be moderate. Those with attachment-associated insecurities are likely to be the least to benefit from current symptom-focused therapies.

Purging disorder: psychopathology and treatment outcomes.

Objective: To characterize a tertiary care treatment-seeking sample and assess post-treatment remission and completion rates for purging disorder (PD).

Method: Consecutively admitted women with PD (n = 122), anorexia nervosa (AN) restricting subtype (AN-R; n = 146), AN binge-purge subtype (AN-B; n = 154), and bulimia nervosa-purging subtype (BN-P; n = 415) were compared on general and eating disorder specific psychopathology. A subsample (n = 256) attended a day treatment program and were assessed for post-treatment remission and completion rates.

Mosaic caveolin-3 expression in acquired rippling muscle disease without evidence of myasthenia gravis or acetylcholine receptor autoantibodies.

Inherited rippling muscle disease is an autosomal dominant disorder usually associated with caveolin-3 mutations. Rare cases of acquired rippling muscle disease with abnormal caveolin-3 localisation have been reported, without primary caveolin-3 mutations and in association with myasthenia gravis and acetylcholine receptor autoantibodies, or thymoma. We present three new patients with electrically-silent muscle rippling and abnormal caveolin-3 localisation, but without acetylcholine receptor autoantibodies, or clinical or electrophysiological evidence of myasthenia gravis.

Testing a maintenance model for eating disorders in a sample seeking treatment at a tertiary care center: a structural equation modeling approach.

Fairburn et al (Fairburn, CG, Cooper, Z, Shafran, R. Behav Res Ther 2003;41:509-528) proposed additional maintenance mechanisms (ie, interpersonal difficulties, mood intolerance, low self-esteem, and perfectionism) for some individuals with eating disorders in addition to core eating disorder psychopathology (ie, overevaluation of eating, weight, and shape and their control). This is the first study to both elaborate and test this maintenance model as a structural model.

CD8(+) T cells in facioscapulohumeral muscular dystrophy patients with inflammatory features at muscle MRI.

Facioscapulohumeral muscular dystrophy (FSHD) is an inherited disease, and although strongly suggested, a contribution of inflammation to its pathogenesis has never been demonstrated. In FSHD patients, we found by immunohistochemistry inflammatory infiltrates mainly composed by CD8(+) T cells in muscles showing hyperintensity features on T2-weighted short tau inversion recovery magnetic resonance imaging (T2-STIR-MRI) sequences. Therefore, we evaluated the presence of circulating activated immune cells and the production of cytokines in patients with or without muscles showing hyperintensity features on T2-STIR-MRI sequences and from controls.

Altruism motivates participation in a therapeutic HIV vaccine trial (CTN 173).

This is the first study examining motivation to participate in an HIV therapeutic vaccine trial of Remune and ALVAC. Trial participants (N=49) completed psychological measures at baseline. While 69% reported some personal risk in participating, 100% felt hopeful for societal benefits.

Mixed connective tissue disease presenting as a peculiar myositis with poor muscle regeneration.

Mixed connective tissue disease (MCTD) is a rheumatological disease which has to be distinguished from other entities causing inflammatory myopathy. The usual clinical presentation of inflammatory myopathy associated with connective tissue disease is not different from isolated polymyositis or dermatomyositis, i.e.

Depressive symptoms are associated with medication use and lower health-related quality of life in overweight women with binge eating disorder.

Background: Depression is a most burdensome illness, with personal and societal costs surpassing those of any other illness. Furthermore, depression affects women at a much higher rate than men. The most prevalent eating disorder among adult women is binge eating disorder (BED).

Attachment insecurity predicts eating disorder symptoms and treatment outcomes in a clinical sample of women.

We examined the extent to which attachment insecurity was related to eating disorder (ED) symptoms, and predictive of treatment outcomes. Women diagnosed with anorexia nervosa (AN) restricting subtype (ANR), AN binge purge subtype (ANB), or bulimia nervosa (BN) completed an attachment scale pretreatment, and ED symptom scales pretreatment (N = 243) and post-treatment (N = 157). A comparison sample of 126 non-ED women completed attachment scales on 1 occasion.

An Italian case of hereditary myopathy with early respiratory failure (HMERF) not associated with the titin kinase domain R279W mutation.

Hereditary myopathy with early respiratory failure (HMERF) is a rare disorder characterized by severe respiratory involvement at onset, muscle weakness starting in the early adulthood, and cytoplasmic bodies with peculiar immunohistochemical reactivity on muscle biopsy. Here we describe a patient who presented with hypercapnic coma at age 32. A detailed light and electron microscopy analysis on muscle biopsy was performed and, together with clinical data, led to the diagnosis.

High HIV knowledge relates to low stigma in pharmacists and university health science students in Guyana, South America.

Objectives: The objective of this study was to investigate HIV knowledge and its relation to HIV stigma among health science students and pharmacists in Guyana, South America. This study also evaluated how the Public Health Strengthening in Guyana Project's (PHSGP) HIV/AIDS/STI educational initiatives influenced HIV knowledge levels among health science students at the University of Guyana.

Methods: A total of 119 individuals, consisting of pharmacists (n=42) and health science students (n=77) participated in this cross-sectional study.

Three-level multilevel growth models for nested change data: a guide for group treatment researchers.

Researchers have known for years about the negative impact on Type I error rates caused by dependencies in hierarchically nested and longitudinal data. Despite this, group treatment researchers do not consistently use methods such as multilevel models (MLMs) to assess dependence and appropriately analyse their nested data. The goals of this study are to review some of the study design issues with regard to hierarchically nested and longitudinal data, discuss MLMs for assessing and handling dependence in data, and present a guide for developing a three-level growth MLM that is appropriate for group treatment data, design, and research questions.

Multilevel modeling of longitudinal data for psychotherapy researchers: II. The complexities.

The authors previously reviewed the basic elements and steps to building multilevel models (MLMs) for longitudinal data typically found in psychotherapy research. The objective of this article is to focus on complexities associated with the MLM for longitudinal data analysis in psychotherapy research, which may result in proper use or misuse of the modeling structure. To do so, the authors illustrate complex scenarios and discuss issues in the implementation and interpretation of the MLM: (a) impact of missing data in the MLM, (b) determination of the complexity of the covariance structure and its implication on model interpretation, (c) issues with centering, (d) model diagnostics for MLM, (e) model formation, including implementation dependent on the treatment of time and distribution of outcome, and (f) model estimation.

Increasing public awareness about hepatitis C: development and validation of the brief hepatitis C knowledge scale.

Hepatitis C virus (HCV) is silently becoming a major public health problem. Currently, no validated HCV knowledge measures exist. This study aimed to develop and validate a brief measure to assess general knowledge about HCV risk factors, modes of transmissions, and treatment options.

Progressive multifocal leukoencephalopathy in a patient with Franklin disease and hypogammaglobulinemia.

We report an association between histologically confirmed progressive multifocal leukoencephalopathy (PML) and an extremely rare humoral immunodeficiency disease, Franklin disease. In our patient, clinical presentation has been typical and prompted us, together with radiological findings, to perform a brain biopsy to confirm the diagnosis even if there was no evidence of any other risk factor except hypogammaglobulinemia. We suggest that PML should be suspected in patients in whom immunosuppression is not obvious (i.

Personality dimensions among women with an eating disorder: towards reconceptualizing DSM.

Objective: To evaluate the incremental validity of a dimensional assessment of personality, after controlling for diagnostic category, in accounting for meaningful variation in eating disorder attitudes and behaviours and in current affective distress among a clinical sample of eating disordered women.

Methods: 244 treatment seeking eating disordered women and 116 non-eating disordered women were assessed with the NEO five factor inventory (NEO-FFI), and with measures of eating disorder attitudes and of affective distress using a cross sectional design.

Results: As predicted, differences were found between eating disordered and non-eating disordered women on several NEO-FFI scales, which provided a context for subsequent analyses.

Multilevel modeling of longitudinal data for psychotherapy researchers: I. The basics.

Psychotherapy researchers are often interested in change or development over time (i.e., pre- to posttreatment to follow-up or the development of process variables across multiple sessions).

Psychometric properties of self-monitoring of eating disorder urges among treatment seeking women: ecological momentary assessment using a daily diary method.

Objectives: The psychometric properties of an ecological momentary assessment (EMA) method used to evaluate eating disorder (ED) urges were examined.

Methods: Participants, 139 women who sought treatment for an ED, completed a daily diary measuring ED urges after each meal for a three week period at the start of treatment, and a measure of ED attitudes and behaviors pre- and post-treatment.

Results: The construct validity of this method was indicated by a two factor solution representing binge eating urges and ED compensatory behavior urges, and by significant differences between ED diagnostic groups on ED urge type.